Partial splenectomy for Gaucher's disease

• Published in: Annals of surgery Vol. 205; no. 6; pp. 693 - 699
• Main Authors: RODGERS, B. M, TRIBBLE, C, JOOB, A
• Format: Conference Proceeding Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott 01-06-1987
• Subjects: Biological and medical sciences; Child; Child, Preschool; Errors of metabolism; Female; Gaucher Disease - therapy; Humans; Lipids (lysosomal enzyme disorders, storage diseases); Male; Medical sciences; Metabolic diseases; Splenectomy; Human; Splenectomy; Gaucher disease; Partial; Lipids; Metabolic diseases; Genetic disease
• ISSN: 0003-4932; 1528-1140
• DOI: 10.1097/00000658-198706000-00011

Gaucher's disease is an autosomal recessive disorder caused by deficiency of beta glucocerebrosidase, resulting in an accumulation of glucocerebroside in the reticuloendothelial system. These patients have massive splenomegaly and bone pain, but may have normal life expectancy. Traditionally, splenectomy has been used to treat hypersplenism, but may be associated with a high incidence of postsplenectomy sepsis and accelerated hepatic and bone lipid deposition. Two children are reported who had partial splenectomy for symptoms of Gaucher's disease. Both patients had laboratory evidence of hypersplenism. A 90% splenectomy was performed, and the residual splenic fragment was wrapped in Vicryl mesh. Both patients are currently asymptomatic with normal hematologic parameters. Postoperative radionuclide scans demonstrate increase in the size of the residual splenic fragment. Partial splenectomy may benefit patients with Gaucher's disease, but long-term follow-up care is necessary.